scholarly journals Hemorrhagic Skull Base Chordoma Presenting As Chordoma Apoplexy

Cureus ◽  
2021 ◽  
Author(s):  
Ece Uysal ◽  
Michael A Cohen ◽  
Hussam Abou-Al-Shaar ◽  
Cheryl Palmer ◽  
William Couldwell
Keyword(s):  
Skull Base ◽  
Skull Base Chordoma

scholarly journals RARE-32. PEDIATRIC METASTATIC SKULL BASE CHORDOMA WITH TP53 MUTATION – A CASE REPORT AND REVIEW OF THE LITERATURE

2020 ◽  
Vol 22 (Supplement_3) ◽  
pp. iii449-iii449
Author(s):  
Akira Shimada ◽  
Kazuhiko Kurozumi ◽  
Kiichiro Kanamitsu ◽  
Hisashi Ishida Ishida ◽  
Kaori Fujiwara ◽  
...  
Keyword(s):  
Skull Base ◽  
Tp53 Mutation ◽  
Cranial Nerves ◽  
Gamma Knife Radiosurgery ◽  
Biological Behavior ◽  
Maximum Diameter ◽  
Cervical Lymph Nodes ◽  
First Choice ◽  
Systemic Metastasis ◽  
Skull Base Chordoma

Abstract Chordoma is an uncommon bone tumor arising from notochordal remnant, which accounts for 1–4% of all bone malignancies. It commonly occurs along the cranial-spinal axis, and skull base is one of most frequent sites, representing 35–49% of all chordoma cases. Surgical resection is widely accepted as the first choice of treatment. There are only limited number of reports about pediatric chordoma cases, and its biological behavior including genetic backgrounds were largely unknown. Here, we present a 5 year-old girl with a large aggressive skull base chordoma of 6 cm in maximum diameter, which eventually had multiple systemic metastasis. We initially tried chemotherapy based on the protocol for the osteosarcoma, but in vain. Because the tumor was highly vascularized on angiography, after embolization of the feeding arteries and bilateral internal maxillary arteries, endoscopic endonasal surgery was performed. The tumor was sufficiently removed, achieving effective mass reduction, and the residual tumors involving the lower cranial nerves and craniocervial junction were additionally treated with Gamma Knife radiosurgery. However, one month later, it showed systemic metastasis to bilateral cervical lymph nodes and lung. We tried chemotherapy with nivolmab and imatinib for this patient, whereas they showed the partial effect. The genetic analysis revealed somatic TP53 c.569C>T, (p.P190L) mutation in chordoma specimen. In the past literature, we found only one study of the adult chordoma cases, in which majority of the patients had somatic TP53 mutation (p.P72R). Further investigation with large number of the cases is essential to clarify the molecular biology of pediatric chordomas.

scholarly journals Radiomics and Dosiomics for Predicting Local Control after Carbon-Ion Radiotherapy in Skull-Base Chordoma

Cancers ◽  
2021 ◽  
Vol 13 (2) ◽  
pp. 339
Author(s):  
Giulia Buizza ◽  
Chiara Paganelli ◽  
Emma D’Ippolito ◽  
Giulia Fontana ◽  
Silvia Molinelli ◽  
...  
Keyword(s):  
Prognostic Factors ◽  
Skull Base ◽  
Local Control ◽  
Tumour Progression ◽  
Risk Groups ◽  
Carbon Ion Radiotherapy ◽  
Survival Models ◽  
Carbon Ion ◽  
Combined Models ◽  
Skull Base Chordoma

Skull-base chordoma (SBC) can be treated with carbon ion radiotherapy (CIRT) to improve local control (LC). The study aimed to explore the role of multi-parametric radiomic, dosiomic and clinical features as prognostic factors for LC in SBC patients undergoing CIRT. Before CIRT, 57 patients underwent MR and CT imaging, from which tumour contours and dose maps were obtained. MRI and CT-based radiomic, and dosiomic features were selected and fed to two survival models, singularly or by combining them with clinical factors. Adverse LC was given by in-field recurrence or tumour progression. The dataset was split in development and test sets and the models’ performance evaluated using the concordance index (C-index). Patients were then assigned a low- or high-risk score. Survival curves were estimated, and risk groups compared through log-rank tests (after Bonferroni correction α = 0.0083). The best performing models were built on features describing tumour shape and dosiomic heterogeneity (median/interquartile range validation C-index: 0.80/024 and 0.79/0.26), followed by combined (0.73/0.30 and 0.75/0.27) and CT-based models (0.77/0.24 and 0.64/0.28). Dosiomic and combined models could consistently stratify patients in two significantly different groups. Dosiomic and multi-parametric radiomic features showed to be promising prognostic factors for LC in SBC treated with CIRT.

Prognostic Factors in Skull Base Chordoma: A Systematic Literature Review and Meta-Analysis

2018 ◽  
Vol 109 ◽  
pp. 307-327 ◽  
Author(s):  
Ming-Xiang Zou ◽  
Guo-Hua Lv ◽  
Qian-Shi Zhang ◽  
Shao-Fu Wang ◽  
Jing Li ◽  
...  
Keyword(s):  
Prognostic Factors ◽  
Literature Review ◽  
Skull Base ◽  
Systematic Literature Review ◽  
Meta Analysis ◽  
Skull Base Chordoma

scholarly journals Tumour seeding in the surgical pathway after resection of skull base chordoma

2016 ◽  
Vol 21 (4) ◽  
pp. 407-411 ◽  
Author(s):  
Marco Krengli ◽  
Arturo Poletti ◽  
Eleonora Ferrara ◽  
Piero Fossati
Keyword(s):  
Skull Base ◽  
Surgical Pathway ◽  
Skull Base Chordoma ◽  
Tumour Seeding

Skull Base Chordoma and Chondrosarcoma: Influence of Clinical and Demographic Factors on Prognosis: A SEER Analysis

2014 ◽  
Vol 82 (5) ◽  
pp. 806-814 ◽  
Author(s):  
Leif-Erik Bohman ◽  
Matthew Koch ◽  
Robert L. Bailey ◽  
Michelle Alonso-Basanta ◽  
John Y.K. Lee
Keyword(s):  
Skull Base ◽  
Demographic Factors ◽  
Skull Base Chordoma

scholarly journals High Red Cell Distribution Width Independently Predicts Adverse Survival in Patients with Newly Diagnosed Skull Base Chordoma

2021 ◽  
Vol Volume 14 ◽  
pp. 5435-5445
Author(s):  
Mingxuan Li ◽  
Yutao Shen ◽  
Yujia Xiong ◽  
Jiwei Bai ◽  
Shuai Wang ◽  
...  
Keyword(s):  
Skull Base ◽  
Red Cell Distribution Width ◽  
Red Cell ◽  
Cell Distribution ◽  
Newly Diagnosed ◽  
Distribution Width ◽  
Skull Base Chordoma

SNF5 as a prognostic factor in skull base chordoma

2017 ◽  
Vol 137 (1) ◽  
pp. 139-146 ◽  
Author(s):  
Mingxuan Li ◽  
Yixuan Zhai ◽  
Jiwei Bai ◽  
Shuai Wang ◽  
Hua Gao ◽  
...  
Keyword(s):  
Prognostic Factor ◽  
Skull Base ◽  
Skull Base Chordoma

Lateral Skull Base Chordoma Mimicking a Paraganglioma

2021 ◽  
Vol Publish Ahead of Print ◽  
Author(s):  
Vir Patel ◽  
Tiffany P. Hwa ◽  
Adam C. Kaufman ◽  
Rachel A. Kolster ◽  
Douglas C. Bigelow
Keyword(s):  
Skull Base ◽  
Lateral Skull Base ◽  
Skull Base Chordoma
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